Enucleation vs Ophthalmic Artery Chemosurgery for Advanced Intraocular Retinoblastoma: A Retrospective Analysis.

Importance: Ophthalmic artery chemosurgery (OAC) has emerged as a primary treatment for advanced-stage retinoblastoma. To our knowledge, the incidence of orbital recurrence in eyes treated with OAC has not been described.
Objective: To determine the incidence of orbital recurrence following enucleation or OAC as primary treatments for advanced-stage retinoblastoma.
Design, Setting, and Participants: Single-institution cohort study with retrospective record review at an academic ophthalmic oncology practice. A total of 140 eyes in 135 patients who presented between February 14, 2006, and March 4, 2014, and were classified as having Reese-Ellsworth group 5 or International Classification of Retinoblastoma (Children's Oncology Group) group D or E retinoblastoma were included; 63 patients (63 eyes) were primarily treated with enucleation and 72 patients (77 eyes) were primarily treated with OAC. This analysis was conducted between August 1, 2014, and March 1, 2015.
Main Outcomes and Measures: Incidence of and time to orbital recurrence, metastasis, and death.
Results: There were 5 orbital recurrences (incidence, 7.9%) in the primary enucleation group and 1 orbital recurrence (incidence, 1.3%) in the primary OAC group during median follow-up times of 42.6 months (range, 6.2-97.1 months) and 38.7 months (range, 9.0-104.3 months), respectively. The 24-month Kaplan-Meier estimate for orbital recurrence-free survival was worse for the enucleation group (92.1%; 95% CI, 82.0-96.7) than for the OAC group (100%) (log-rank test, P = .049). The enucleation group had 5 cases of metastatic disease (7.9%) and 2 deaths (3.2%). In the OAC group, there were 3 cases of metastatic disease (4.2%) and no deaths. Kaplan-Meier analysis of metastasis-free survival and overall survival yielded no differences between the 2 treatment groups. Analysis of a number of features of the 2 groups revealed more eyes with iris neovascularization in the enucleation group (25.4%) than in the OAC group (5.2%) and more eyes with group E retinoblastoma in the enucleation group (87.3%) than in the OAC group (29.9%), although neither of these factors was an independent predictor of orbital relapse in a Cox proportional hazards model.
Conclusions and Relevance: In this single-institution retrospective study of advanced intraocular retinoblastoma, there were more orbital recurrences in the group primarily treated with enucleation. Ophthalmic artery chemosurgery for advanced intraocular retinoblastoma was not found to increase the chance of orbital recurrence, metastatic disease, or death compared with primary enucleation.